Overview
Definition
A chronic condition characterized by irreversible, abnormal dilation and destruction of the bronchial walls, leading to impaired mucociliary clearance and recurrent infections.
Epidemiology
Prevalence increases with age. Often a final common pathway of severe recurrent lung insults.
Etiology & Risk Factors
- Cystic Fibrosis (most common cause in developed nations)
- Post-infectious (severe pneumonia, Tuberculosis, Pertussis)
- Primary Ciliary Dyskinesia (Kartagener syndrome)
- Immunodeficiencies (Common Variable Immunodeficiency - CVID)
- Allergic Bronchopulmonary Aspergillosis (ABPA)
Clinical Symptoms
- Chronic cough with massive amounts of purulent, foul-smelling sputum
- Hemoptysis
- Dyspnea and wheezing
- Fatigue and weight loss
Clinical Approach
Diagnosis
- High-Resolution CT (HRCT) of the chest (Diagnostic): Dilated airways larger than accompanying blood vessel ('Signet ring sign'), 'Tram-track' lines
- PFTs: Obstructive pattern
- Sputum culture (often positive for Pseudomonas or H. influenzae)
Management
- Airway clearance (Chest PT, flutter valves)
- Aggressive antibiotic therapy for acute exacerbations (guided by sputum cultures)
- Macrolides (Azithromycin) 3x/week for anti-inflammatory properties and to reduce exacerbations
- Bronchodilators
- Embolization (if massive life-threatening hemoptysis occurs)
Complications
- Massive hemoptysis
- Cor Pulmonale
- Respiratory failure
- Secondary Amyloidosis (AA)